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Year : 2010  |  Volume : 53  |  Issue : 4  |  Page : 906-907
Malignant peripheral nerve sheath tumor of the tongue

1 Department of Pathology, Shiraz University of Medical Sciences, Shiraz, Iran
2 Transplant Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
3 Department of Otolaryngology, Shiraz University of Medical Sciences, Shiraz, Iran

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Date of Web Publication27-Oct-2010

How to cite this article:
Ashraf M J, Azarpira N, Khademi B, Maghbul M. Malignant peripheral nerve sheath tumor of the tongue. Indian J Pathol Microbiol 2010;53:906-7

How to cite this URL:
Ashraf M J, Azarpira N, Khademi B, Maghbul M. Malignant peripheral nerve sheath tumor of the tongue. Indian J Pathol Microbiol [serial online] 2010 [cited 2021 Aug 5];53:906-7. Available from: https://www.ijpmonline.org/text.asp?2010/53/4/906/72073


The malignant peripheral nerve sheath tumors (MPNSTs) arise either in association with peripheral nerves or within preexisting neurofibromas. They account for approximately 5-10% of all soft tissue sarcomas, arise from Schwann cells with a strong association with type I neurofibromatosis (NF1), but sporadic cases may occur. If associated with NF1, the tumor tends to be present at a younger age (28-36 years) than the sporadic cases (40-44 years). The proximal extremities are the most common locations of these tumors. This is an aggressive tumor with a poor prognosis despite wide excision, chemotherapy, and radiotherapy. About 8-16% MPNSTs develop in the head and neck region. There are few reports of sporadic cases involving the tongue in the English literature. [1],[2],[3],[4]

A 67-year-old woman was referred to the Otolaryngology Department of Shiraz University of Medical Sciences, in February 2009, complaining of swelling in the tongue for 1 week. The patient had no stigmata of neurofibromatosis. The intraoral physical exam revealed a palpable 4 × 3 cm, nonulcerative mass. Computed tomographic (CT) scan showed a soft tissue mass, in the base of the tongue that extended to the retropharyngeal space [Figure 1]. Fine needle aspiration (FNA) of tongue mass was performed. Microscopic examination of the smeared aspirate showed highly cellular smears composed of clusters resembling interlacing fascicles. The individual cells revealed oval-to-spindle nuclei, with an ill--defined cytoplasmic border [Figure 2]. Mitosis was found with careful searching. The tumor rendered as malignant spindle cell tumor with a mesenchymal origin.
Figure 1: MRI scan revealed a soft tissue mass arising from the tongue

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Figure 2: Cellular smear composed of spindle-to-oval shaped cells with pleomorphism and a high N:C ratio (Wright-Giemsa stain, ×400)

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Tumors such as MPNST, leiomyosarcoma, rhabdomyosarcoma, fibrosarcoma, monophasic variant of synovial sarcoma, and spindle cell variant of squamous cell carcinoma are considered in differential diagnosis. [5] The patient underwent hemiglossectomy. In histological sections, the tumor is composed of hyperchromatic spindle cells growing in a fascicular pattern. The architecture arranged in alternating hypocellular and densely cellular areas with increased mitotic activity (1-2 mitosis/10 HPF) [Figure 3]. A focus of necrosis was present. For definite diagnosis, the sections were immunostained for the following panel of antibodies: vimentin; S100; pancytokeratim (AE1/AE3); epithelial membrane antigen (EMA); smooth muscle actin (SMA); desmin; neuron-specific enolase (NSE) (DAKO Company, Denmark). Among these markers, only positive vimentin and weakly focal positivity for S100 [Figure 4] and NSE were observed, which was in favor of the neurogenic origin of this tumor. The lack of expression of keratin and EMA were against a carcinoma and synovial sarcoma. It expressed both S100 and vimentin and this argued against a diagnosis of fibrosarcoma. In IHC, focal S100 protein is positive in 50-90% of MPNSTs, and positivity of Leu-7 and myelin basic protein also is helpful for diagnosis of the MPNSTs.
Figure 3: High-grade sarcoma infiltrated between muscle fibers (H and E, ×400)

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Figure 4: Focal S100 positivity in tumoral cells (IHC, ×400)

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English literature review about MPNSTs of the tongue demonstrated five well-documented cases, not associated with type I neurofibromatosis. [1],[2],[3],[4] All of them were young (6-37 years), but our patient is in the sixth decade. Wide surgical excision is a choice treatment, but local recurrence is common. Hematogenous metastasis occurs in at least half of all cases. [3],[4]

Factors such as size of the lesion, adequacy of surgical margins, and association with type I neurofibromatosis affect the patient's survival. Our case is sporadic and was managed with wide surgical excision. The patient remains free of disease after 8 months and the prognosis seems to be good.

   References Top

1.Hemalatha AL, Karthikeyan TM, Bharatnur SS, Kumar AS. Malignant peripheral nerve sheath tumor in oral cavity, a rare site. Indian J Pathol Microbiol 2006;49:397-9.  Back to cited text no. 1
2.Kenali MS, Bridger PG, Baldwin M, Smee R. Malignant peripheral nerve sheath tumour of the tongue. Aust N Z J Surg 1999;69:243-6.  Back to cited text no. 2
3.Piatelli A, Angelone A, Pizzicannella G, Piatelli M. Malignant schwannoma of the tongue. Report of a case and review of the literature. Acta Stomatol Belg 1984;81:213-25.  Back to cited text no. 3
4.Yamazaki H, Tsukinoki K, Shimamura K, Kaneko A. Malignant peripheral nerve sheath tumor with perineurial cell differentiation arising from the tongue. Oral Oncol Extra 2005;41:77-80.   Back to cited text no. 4
5.Gupta K, Dey P, Vashisht R. Fine-needle aspiration cytology of malignant peripheral nerve sheath tumors. Diagn Cytopathol 2004;31:1-4.  Back to cited text no. 5

Correspondence Address:
N Azarpira
Organ Transplant Research Center, Zand Street, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz - 7193711351
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.72073

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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